Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience

Seyoung Seo; Changhoon Yoo; Dok Hyun Yoon; Shin Kim; Jung Sun Park; Chan-Sik Park; Jooryung Huh; Cheolwon Suh

doi:10.5045/br.2014.49.4.253

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Original Article

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Blood Res 2014; 49(4):

Published online December 31, 2014

https://doi.org/10.5045/br.2014.49.4.253

Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience

Seyoung Seo¹, Changhoon Yoo², Dok Hyun Yoon², Shin Kim², Jung Sun Park², Chan-Sik Park³, Jooryung Huh³, and Cheolwon Suh^2*

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¹Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

²Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

³Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Correspondence to : Correspondence to Cheolwon Suh, M.D., Ph.D. Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, Korea. Tel: +82-2-3010-3209, Fax: +82-2-3010-6961, csuh@amc.seoul.kr

Received: September 12, 2014; Revised: October 30, 2014; Accepted: November 14, 2014

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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Abstract

Background

Multicentric Castleman's disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD.

Methods

Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis.

Results

Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival.

Conclusion

Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors.

Keywords Multicentric Castleman's disease, Giant lymph node hyperplasia, Angiofollicular lymphoid hyperplasia, Prognosis, HIV

Article

Original Article

Blood Res 2014; 49(4): 253-258

Published online December 31, 2014 https://doi.org/10.5045/br.2014.49.4.253

Clinical features and outcomes in patients with human immunodeficiency virus-negative, multicentric Castleman's disease: a single medical center experience

Seyoung Seo¹, Changhoon Yoo², Dok Hyun Yoon², Shin Kim², Jung Sun Park², Chan-Sik Park³, Jooryung Huh³, and Cheolwon Suh^2*

¹Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

²Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

³Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Correspondence to: Correspondence to Cheolwon Suh, M.D., Ph.D. Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 138-736, Korea. Tel: +82-2-3010-3209, Fax: +82-2-3010-6961, csuh@amc.seoul.kr

Received: September 12, 2014; Revised: October 30, 2014; Accepted: November 14, 2014

Abstract

Background

Methods

Results

Conclusion

Keywords: Multicentric Castleman's disease, Giant lymph node hyperplasia, Angiofollicular lymphoid hyperplasia, Prognosis, HIV

Abstract

Fig 1.

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Figure 1.

The Kaplan-Meier depicting overall survival. (A) Extravascular fluid accumulation symptoms and signs. (B) Disease extent using the Ann Arbor staging system.

Blood Research 2014; 49: 253-258https://doi.org/10.5045/br.2014.49.4.253

Table 1 . Baseline characteristics of patients..

Abbreviation: POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma-proliferative disorder, and skin changes..

Table 2 . Laboratory data..

^a)Patients with abnormal laboratory findings/total tested patients..

^b)Each patient had a different subtype; IgG λ, IgM λ, IgA λ..

Abbreviations: β2MG, beta-2 microglobulin; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; Ig, immunoglobulin; LDH, lactate dehydrogenase..

Table 3 . Initial treatment and best response..

^a)Rituximab was added from the second to sixth cycle in a patient with Evan's syndrome; this patient achieved CR..

^b)A patient received 1 cycle of rituximab plus CHOP followed by melphalan and prednisolone..

Abbreviations: CHOP, cyclophosphamide, adriamycin, vincristine, prednisolone; CR, complete response; CVP, cyclophosphamide, vincristine, prednisolone; INF-α, interferon-alpha; NE, not evaluable; PD, progressive disease; PR, partial response; R-CHOP, rituximab plus CHOP; SD, stable disease..

Table 4 . Univariate analysis for overall survival according to log-rank tests..

Abbreviation: OS, overall survival..